Gorlin-Goltz syndrome is an autosomal dominant disorder characterized by exteremly pleomorphic clinical manifestations including multiple odontogenic keratocysts. These cysts constitute only 3% to 11% of all jaw cysts, but are often of great concern to clinicians because of high recurrence rate, aggressive behavior and association with Gorlin-Goltz syndrome as they are found in 75-80% of the affected patients. In the paper there were presented histological, clinical, and radiological features of odontogenic keratocyst in Gorlin-Goltz syndrome.
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